نتایج جستجو برای: protein misfolding
تعداد نتایج: 1235138 فیلتر نتایج به سال:
Inherited missense mutations in the myocilin olfactomedin domain are causative for early-onset glaucoma, via a toxic gain of function. We have elaborated atomic-level details misfolding relevant to glaucoma pathogenesis. Pathogenic olfactomedin-resident mutations, which account 90% all documented disease variants, destabilized and undergo templated amyloid-like aggregation under mild physiologi...
A common characteristic of familial (fALS) and sporadic amyotrophic lateral sclerosis (sALS) is the accumulation aberrant proteinaceous species in motor neurons spinal cord ALS patients—including aggregates human superoxide dismutase 1 (hSOD1). hSOD1 an enzyme that occurs as a stable dimeric protein with several post-translational modifications such formation intramolecular disulfide bond acqui...
The accumulation of misfolded proteins in the human brain is one of the critical features of many neurodegenerative diseases, including Alzheimer's disease (AD). Assembles of beta-amyloid (Aβ) peptide-either soluble (oligomers) or insoluble (plaques) and of tau protein, which form neurofibrillary tangles, are the major hallmarks of AD. Chaperones and co-chaperones regulate protein folding and c...
The solvation shell is essential for the folding and function of proteins, but how it contributes to protein misfolding aggregation has still be elucidated. We show that mobility H2O molecules influences rate amyloid α-synuclein (αSyn), a associated with Parkinson's disease. When within reduced by presence NaCl, αSyn increases. Conversely, in CsI increased reduced. Changing solvent from D2O lea...
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